Conditions 2 Flashcards
(100 cards)
1
Q
What are the characteristics of febrile seizures? (are they harmful?)
A
- Generalised tonic-clonic seizures, dont last long.
- Period of postictal drowsiness
- Periods of cyanosis/apnoea.
Majority are harmless! 30-40% will develop further febrile seizures.
2
Q
What are atypical febrile seizures?
A
> 15mins, or >2 within 24hrs, or a focal seizure.
^risk of epilepsy
3
Q
Management of a febrile seizure?
A
TIME it, + prevent child from hurting themselves (don’t restrain)
- > 5mins = Buccal midazolam
- Another 10mins= IV Lorazepam
- > 30mins= treat as status epilepticus
4
Q
What are the 2 types of Breath Holding Attacks in toddlers?
A
- Cyanotic spells (breath holding episode)
2. Pallid spells (reflex anoxic seizure)
5
Q
What is the difference of a breath-holding episode + a reflex anoxic seizure?
A
- Breath-holding: precipitated by anger/crying > holds breath > goes blue > then limp, rapid recovery (absence of postictal phase)
- Reflex anoxic: precipitated by pain/minor injury > triggers vagal reflex + stops breathing > goes pale > brief seizure sometimes > rapid recovery (absence of postictal phase)
6
Q
What are generalised epileptic seizures?
A
Discharge arises from both hemispheres.
- Tonic-clonic (grand mal)
- Absence (petit mal)
- Myoclonic
7
Q
1) Tonic clonic seizures?
2) Absence seizures?
3) Myoclonic seizures?
A
1) Tonic: LoC, limbs extend, back arches + breathing stops. Teeth clenched. Clonic: Intermittent jerking, irregular reathing + micturition. Postictal depression.
2) Fleeting impairments of consciousness, child assumed daydreaming.
3) Shock-like jerks, can result in sudden falls. Common in children with structural neurological disorder.
8
Q
What are some types of focal epileptic seizures?
A
- Simple partial: twitching/jerking one side of face/arm/leg. Consciousness retained. Jacksonian march. Sometimes progresses to full tonic-clonic.
- Complex partial: altered consciousness a/w strange sensations, hallucinations. Commonly chewing, sucking + swallowing movements. Postictal phase.
9
Q
What is a Jacksonian march?
A
When jerking starts in one part of the body and spreads.
10
Q
What are infantile spasms? (West Syndrome!)
A
Form of myoclonic epilepsy but considered separately as have particularly poor prognosis.
11
Q
Presentation of Infantile spasms?
A
- Age of onset 3-8months
- Typical flexion spasms (knife jerk/ ‘salam’ spasms) lasting few secs, in clusters up to 30mins.
- Usually occur on waking from sleep.
- ?Hx perinatal complications (meningitis/asphyxia)
- Following onset= developmental regression.
- EEG characteristic ‘hyperrhythmic’ (chaotic) pattern.
12
Q
What genetic association is a/w infantile spasms?
A
SCN1A mutation!!!
13
Q
EEG is indicated when epilepsy is suspected. What are the characteristic patterns for:
- Simple absence (petit mal) seizures
- Infantile spasms (West syndrome)
A
- Three per second spike + wave discharge, B/L synchronous.
2. Hypsarrhythmia, + chaotic background of slow-wave activity.
14
Q
Management of Infantile Spasms?
A
- Vigabatrin (1st line)
- ACTH hormone, or prednisolone.
15
Q
What is the 1st line management of all generalised seizures in children?
A
Valproate
- Except in females of child bearing age!!
- Also, if the epilepsy is established + tonic-clonic seizures ONLY then chose : Lamotrigine!
16
Q
What is not recommended in all generalised seizures apart from tonic-clonic?
A
Carbamazepine, gabapentin or phenytoin.
these are NOT recommended in absense/ myoclonic/ tonic/atonic seizures
17
Q
What is generally second line in all generalised seizures?
A
Lamotrigine
18
Q
What is 1st line mx in focal seizures?
A
Lamotrigine!
Also can use: Carbamazepine, Valproate.
19
Q
What are the key s/e of Valproate?
A
- Wt gain, hair loss
- Rare idiosyncratic liver failure (need to monitor LFTs)
- High teratogenic potential (Pregnancy Prevention Program if no alternative)
20
Q
What are the s/e of Carbamazepine/Oxcarbazepine?
A
- Rash, neutropenia, hyponatraemia, ataxia.
- P450 INDUCER!
21
Q
S/e of Topiramate?
A
Nasty!
-Drowsiness, withdrawal, wt loss.
22
Q
S/e of Vigabatrin?
A
- Restriction of visual fields
- Sedation
23
Q
Mx of Acute Seizures in children?
A
- Recovery position. Rectal Diazepam, or Buccal midazolam
- If seizure continues >25mins : phenytoin
- If seizure continues >45mins : anaesthesia
24
Q
Tension headaches:
a) Character
b) Timing
c) Mx
A
a) Constricting, band like. Usually develop towards later childhood.
b) End of day. Stress as home/school.
c) Reassurance, paracetamol. Minimise attention to them!
25
Migraine without aura (90% of migraines):
a) Character
b) Timing
c) Associated features
a) Throbbing/pulsatile over temporal/frontal areas. Usually develop late childhood/early teens.
b) Episodic (1-72hrs)
c) GI disturbances, photo/phonophobia. Aggrivated by physical activity!
26
Management of migraine without aura?
- Rest, simple anaesthesia.
- Diet control
- If frequent: prophylactic propranolol/pizotifen.
- In teens: sumatriptan.
27
What is the character of a migraine with aura?
Headache is preceded by an aura (visual/sensory/motor).
Aura:
-Negative phenomena: hemianopia, scotoma (small areas visual loss)
-Positive phenomena: fortification spectra (zig-zag line)
28
Mx of migraine with aura?
Sleep often relieves.
There is a presence of premonitory sx: tiredness, poor conc., autonomic feats
29
Red Flags in a child with headache
- Acute onset/ severe pain
- Fever
- Intensifies lying down
- A/w vomiting
- Regression of developmental skills
- Consistently U/L pain
- Cranial bruit
- HTN or papilloedema
30
What are some characteristic features of ^ICP or space occupying lesions?
- Worse lying, morning vomiting (without nausea)
- Night time waking
- Change in mood/ personality/ educational performance.
31
Head injury in a child: what advice can be given in a pre-hospital setting?
Advise that child must go to hospital if:
- Any LoC/ seizure
- High energy head injury
- Any focal neurological deficit since the injury
- Any amnesia
- Any vomiting
- Safeguarding concerns
32
What are the signs for a basal skull fracture?
- Panda eyes (peri-orbital bruising)
- Battle's sign (mastoid bruising)
- CSF leaking from nose/ear
33
What is the difference between non-communicating + communicating hydrocephalus?
Non-communicating (obstructive)= obstruction within the ventricular system/ aquaduct.
Communicating= obstruction at the arachnoid villi (site of CSF absorption)
34
Causes of non-communicating hydrocephalus?
- Congenital: aquaduct stenosis, Dandy-Walker malformation (outflow atresia), Chiari malformation.
- Posterior fossa neoplasm
- Intraventricular haemorrhage (in prem)
35
Causes of communicating hydrocephalus? (failure to reabsorb CSF)
- Subarachnoid haemorrhage
| - Meningitis (pneumococcal, TB)
36
What is hydrocephalus commonly a/w?
Neural tube anomalies (occurs in 80% spina bifida)
37
Presentation of hydrocephalus in infants?
| obviously this varies with age + rate in ^ICP
Common: irritability, lethargy, poor appetite, vomiting.
- Accelerated head growth (anterior fontanelle open + bulging, sutures separated)
- Broad forehead, eyes deviated down ('setting sun' sign)
- Spasticity, clonus + brisk deep tendon reflexes
38
Management of hydrocephalus?
- Cranial US/MRI/CT
- Head circumference monitored on centile charts.
- Intracranial shunts (ventriculoperitoneal)
39
Difference between Plagiocephaly and Craniosynostosis?
Plagiocephaly: asymmetric flattening of one side of skull from positional moulding.
Craniosynostosis: premature fusion of one of more sutures, leading to distortion of head shape.
40
What is Sturge-Weber Syndrome associated with?
-Haemangiomatous facial lesion (port-wine stain), in the distribution of the trigeminal nerve (a/w similar lesion intracranially). Always involves the ophthmalmic division.
41
Presentation of Sturge-Weber syndrome?
Most severe: epilepsy, learning disabilities + hemiplegia.
Less severe: deterioration unusual after 5yrs, may still be seizures/learning difficulties.
-^risk glaucoma
42
What medication can be used for Gilles de la Tourette's syndrome? (chronic tic disorder)
-Clonidine or resperidone.
43
What is Duchenne muscular dystrophy?
- Progressive disorder resulting in death in early 20s.
- X-linked recessive, Dystrophic gene defect..
- It is the commonest hereditary neuromuscular disease.
44
Presentation of Muscular Dystrophy?
- Baby boy normal at birth, delayed walking.
- 4-6yrs: frequent falls, lordotic waddling gait.
- Enlarged, weak calves.
- GOWER's SIGN (characteristic)
- Creatine kinase level elevated x10
45
What may a rigid flat foot indicate in older children?
A RIGID flat foot is pathological:
- Tendo-Achilles contracture (ankle)
- Tarsal coalition (lack of segmentation between 1/more bones of foot)
- Juvenile idiopathic arthritis
46
How does Septic Arthritis present in children? (most common <2yrs)
- Joint usually hot, swollen + acutely tender
- +/- fever
- Limited + painful movement of affected joint
- Joint effusion in peripheral joints
- Initial presentation may be a limp
47
Mx of septic arthritis?
- IV flucloxacillin
| - Joint should be splinted in acute stage, then mobilised to prevent permanent deformity
48
What is 'transient synovitis'? (a common DDx of septic arthritis)
Transient synovitis= 'irritable hip', the commonest cause of acute hip pain in children.
2-12yrs, following/accompanying a viral infection.
In a few, TS precedes the development of Perthes disease.
49
What does Reactive Arthritis (commonest form of arthritis in children) usually follow?
Extra-articular infections:
- Children: enteric bacteria (shigella, salmonella, campylobacter)
- Adolescents: viral (chlamydia, gonococcus), mycoplasma + borrelia burgdorferi (Lyme disease).
- Rheum. fever + post-strep in developing countries.
50
What is the presentation of reactive arthritis/irritable hip?
- Low grade fever
- Transient joint swelling (<6wks), usually ankles/knees
- Acute-phase reactants normal/mildly^, XRays normal
- Generally transient + good outcome (NSAIDs only)
51
How does Juvenile Idiopathic Arthritis present?
- Gelling (stiffness after rest)
- Morning joint stiffness + pain
- Initially min. joint swelling, then subsequent swelling + inflammation.
- Chronic: proliferation of synovium + swelling of periarticular soft tissues.
52
What are the long term features of Juvenile Idiopathic Arthritis?
Bone expansion from overgrowth.
Knee: leg lengthening + valgus deformity.
Hands: digit length discrepancy.
53
(types of JIA)
| Characteristics of systemic JIA (Still's disease)?
- Rarest form
- Articular: large + small joints affected
- Extra-articular: remitting fever, macular rash, hepatosplenomegaly, lymphadenopathy, wt loss.
- Lab results: anaemia, ^neutrophils+platelets.
54
(types of JIA)
| Characteristics of Polyarticular JIA?
- Articular: large+small joints affected symmetrically, morning stiffness.
- Extra-articular: chronic anterior uveitis (5%)
55
(types of JIA)
| Characteristics of Pauciarticular/Oligoarticular JIA?
- Commonest form of JRA
- Articular: <5joints, usually large
- Extra-articular: asymmetrical growth, chronic anterior uveitis (20%! NEED REGULAR OPTHALM!)
56
```
What do Rhesus factor/ antinuclear antibodies say in:
a) Systemic
b) Polyarticular
c) Pauci/Oligoarticular
(JRA)
```
a) Negative
b) RhF-ve, ANA+ve
c) RhF-ve, ANA+ve
57
What are systemic corticosteroids (IV methylprednisolone) used to manage?
-Severe polyarthritis as induction agent.
-Systemic arthritis (life-saving)
Avoided if poss to minimise risk of growth suppression + osteoporosis.
58
What is the cause of a Limping Child age 3-5yrs?
Hip effusion- usually caused by transient synovitis or infection.
MUST differentiate from septic arthritis!! (difficult on USS, usually need XR)
59
What is Perthes disease?
Limping, 5-8yrs, M:F 5:1
Idiopathic necrosis of hip (due to interruption in blood supply, followed by revascularisation + reosification over 18-36months).
13% bilateral
60
What are the Xray findings of a child with Perthes disease?
Fragmented flattened femoral head, subsequently becomes fragmented + irregular.
61
Mx of Perthes disease?
- If identified early: bed rest + traction.
- Severe/late: maintain hip in abduction w/plasters or calipers (femoral head covered by acetabulum to act as mould for re-ossifying epiphysis)
62
What is a Slipped Capital Femoral Epiphysis?
Idiopathic fracture through proximal femoral growth plate- results in displacement of epiphysis of femoral head postero-inferiorly.
Limping teenager 12-15yrs, often obese, 1/3 B/L.
63
What is Slipped Capital Femoral Epiphysis associated with?
Metabolic endocrine abnormalities (hypothyroidism + hypogonadism)
64
Presentation of Slipped Capital Femoral Epiphysis?
```
Limp w/hip pain may be referred to knee.
Onset acute (post-trauma) or insidious.
Examination: restricted abduction + internal rotation of hip.
```
65
Recommended isolation for:
a) Measles
b) Mumps
c) Rubella
a) Measles: from onset of catarrhal stage to day 5 of rash.
b) Mumps: until swelling subsides
c) Rubella: none except non-immune women in 1st trimester.
66
What is the recommended isolation for Chicken Pox?
Until all lesions are crusted over.
67
What is involved in the '5 in 1' vaccine?
- Diptheria
- Tetanus
- Pertussis
- H influenzae type b (Hib)
- Polio
68
When should MMR vaccine NOT be given?
- Non-HIV related immunodeficiency.
- Allergy to neomycin/kanamycin.
- Hx of anaphylaxis to egg should be given under supervision.
69
Much of the damage caused by meningeal infection results from the host response. What are 3 pathophysiologies of this?
1. Release of inflam. mediators + activated leukocytes --> endothelial damage --> cerebral oedema, ^ICP + decreased cerebral bloodflow.
2. Inflammatory response --> vasculopathy --> cerebral cortical infarction.
3. Fibrin deposits may block resorption of CSF by arachnoid villi --> hydrocephalus.
70
What are the main organisms causing meningitis in:
a) Neonates - 3months
b) 1month - 6yrs
c) >6yrs
a) Group B strep, E. coli, Listeria monocytogenes.
b) Neisseria meningitidis, Strep pneumoniae, Haemophillus influenzae.
c) Neisseria meningitidis, Strep pneumoniae,
71
What is the difference between where the organism effects in Bacterial + Viral meningitis?
Bacterial: usually confined to meninges.
Virus: may invade underlying brain, causing meningoencephalitis.
72
What types of virus cause meningitis in infants/children?
* Mumps virus
* Coxsackie & echo viruses (enterovirus)
* Herpes simplex
* Adenovirus
* Poliomyelitis (developing countries)
73
General presentation of Meningitis in infants/children?
- Papilloedema rare in children
- Bulging fontanelle late sign in infants.
- Neck stiffness not always present.
- Opisthotonus (arching of back)
74
Viral presentation of Meningitis?
- Preceded by pharyngitis/GI upset
- Then fever, headache + neck stiffness (not reliable sign)
- Classical head retraction a late feature (contrasting to adults)
75
Bacterial presentation of Meningitis?
- Early feature = reduction in consciousness level
- High pitched cry
- Convulsions in infants
- In meningococcal disease: petechial haemorrhages > purpuric rash.
76
What do you assume if a febrile child presents with Purpura?
Assume to be due to meningococcal sepsis, even if child doesn't appear unduly ill at time.
-Give IV BENZYLPENICILLIN + transfer to hospital.
77
Mx of viral/bacterial meningitis?
Viral: usually self-limiting.
Bacterial: IV cefotaxime/ ceftriaxone 10-14days (3rd gen cephalosporins)
-Also dexamethasone beyone neonatal period!
78
What do household contacts of Neisseria meningitidis receive?
```
Prophylactic Rifampicin (along w/infected child after Abx).
Haemophilus influenzae injection.
```
79
Contraindications to a Lumbar puncture in suspected meningitis? (to examine CSF- cloudy in bacterial)
- Signs of raised ICP (papilloedema, low HR, high BP)
- Focal neuro signs
- Cardiorespiratory instability
- Coagulopathy
- Thrombocytopenia
- Local infection at site of LP
80
Describe the following signs that are associated with neck stiffness:
a) Brudzinski sign
b) Kernig sign
a) Brudzinski sign: flexion of neck w/child supine causes flexion of knees + hips.
b) Kernig sign: when child lying supine w/hips + knees flexed, there is back pain on extension of knee.
81
What are the key differences in the CSF between bacterial + viral meningitis?
Colour: cloudy in bacterial, clear in viral.
Protein: ^^in bacterial, ^/-in viral.
Glucose: decreased in bacterial, normal/decreased in viral.
82
What is the commonest long-term complication in VIRAL meningitis?
Sensorineural hearing impairment.
83
What are some complications of BACTERIAL meningitis?
- Hydrocephalus (communicating or non-communicating, when blocked ventricular outlet by fibrin)
- Subdural effusion (a/w haemophilus influenzae + pneumococcal meningitis)
- Hearing loss
- Local vasculitis (may lead to CNS palsies)
- Cerebral infaction (may lead to focal seizures + epilepsy)
- Acute adrenal failure
84
What are some general causes of purpura/easy bruising?
- Thrombocytopenia (^platelet destruction/consumption, impaired platelet production)
- Platelet count normal: vascular disorders (CT disorders, meningococcal infection, Henoch-Schonlein purpura, SLE.
85
Presentation of Immune Thrombocytopenic Purpura?
- Acute onset (1-2wks after viral infection)
- Reduced platelet count, Increased megakaryocytes
- Petechiae + superficial bruising
- Mucosal bleeding
- Child appears clinically well
- Intracranial haemorrhage in 1%
86
Mx of Immune Thrombocytopenic Purpura?
- If mild: none (usually remit spont. 6-8wks)
- Platelet transfusion if v low count
- Supportive mx if becomes chronic ITP (20%)
87
What is Autoimmune thrombocytopenic purpura caused by?
Maternal IgG antibodies crossing placenta + damaging foetal platelets --> foetus becomes thrombocytopenic.
88
What is the pathophysiology of Disseminated Intravascular Coagulation (DIC)?
-Coagulation pathway activation, leading to diffuse fibrin deposition in the microvasculature + consumption of coagulation factors + platelets.
89
What is the commonest cause of activation of coagulation in DIC?
Severe sepsis + shock due to circulatory collapse (e.g. meningococcal septicaemia)
90
What investigation results should point to DIC?
When the following coexist:
- Thrombocytopenia
- Prolonged PTT/ATPP
- Low fibrinogen, raised fibrinogen degradation product
- Raised D-dimer
- Microangiopathic haemolytic anaemia
91
What is Purpura fulminans associated with?
- Mostly: Meningococcal disease (vasculitis- leads to loss of large areas of skin by necrosis).
- Also rarely occurs after VZV infection.
92
What is the commonest cause of septicaemia in neonates?
Group B strep, or Gram -ve organisms (from birth canal)
93
What is the physiology of septicaemia?
Host releases inflammatory cytokines + activates endothelial cells.
(commonest cause in childhood is meningococcal infection, which may/not be accompanied by meningitis)
94
What is the dermatological presentation of chick pox? (varicella)
Rash first appears on trunk/face --> progresses to peripheries.
Papules to Vesicles to Pustules + then crusts over.
95
What is the mx for adolescents/adules with a primary infection of chicken pox?
Oral valaciclovir
96
What is Parvovirus B19 ('slapped-cheek syndrome')?
Causes erythema infectiousum or Fifth disease.
Erythema infectiousum:
Viraemic phase (fever, malaise, headache), then characteristic facial rash, then macpap 'lace' like rash on trunk/limbs.
97
What is the most serious consequence of Parvovirus?
Aplastic crisis- occurs in children with chronic haemolytic anaemias (sick cell/ thalassaemia), and immunodeficiency (malignancy).
98
What is the characteristic presentation of Conjunctivitis from:
a) Gonococcal infection
b) Chlamydia infection?
a) First 48hrs of life: purulent discharge w/conjunctival infection + swollen eyelids. Need to culture + IV cephalosporin.
b) 1-2wks of life: purulent discharge + swollen eyelids. PO erythromycin 2wks.
99
Food allergies can be IgE-mediated and non-IgE mediated, what is the difference?
IgE-mediated: hx of allergic sx from urticaria to anaphylaxis (10mins after ingestion).
Non-IgE mediated: involves GI tract (D+V, abdo pain, failure to thrive), colic/eczema may be present (usually hours after ingestion).
100
Diagnosis of food allergies?
IgE- mediated: skin-prick tests + measure specific IgE Abs in blood (RAST test)
Non-IgE mediated: clinical Hx.
Gold standard (for both): exclusion of relevant food under a dietician's supervision.
